1/303. Spontaneous remission of anemia associated with a myelodysplastic syndrome with disease evolution into a myeloproliferative state. A red cell transfusion-dependent patient with a myelodysplastic syndrome had progression into a myeloproliferative state with thrombocytosis. At the same time, the patient became transfusion independent, and a subsequent bone marrow examination revealed a previously undetected loss of chromosome 7. The patient remains well with control of thrombocytosis by anagrelide therapy. ( info) |
2/303. Portal, mesenteric, and splenic vein thromboses after splenectomy in a patient with chronic myeloid leukemia variant with thrombocythemic onset. Portal, mesenteric, or splenic vein thrombosis is a very uncommon complication with significant mortality in the patients undergoing splenectomy for hematologic disorders. We report a 49-year-old woman who developed portal, superior mesenteric, and splenic vein thromboses after splenectomy. Four years before the event, she presented with a marked thrombocytosis and was diagnosed to have chronic myeloid leukemia variant with thrombocythemic onset as evidence by philadelphia (Ph1) chromosome and a b3a2 BCR/ABL transcript. Six weeks after splenectomy, she developed severe epigastric pain. The diagnosis of thromboses of portal, mesenteric, and splenic veins was made by computed tomography scan and Doppler sonogram. She was successfully treated with antegrade intraarterial urokinase therapy via superior mesenteric artery and long-term anticoagulant therapies. To our knowledge, our patient is the first case of portal, mesenteric, and splenic vein thromboses after splenectomy in a patient with CML variant with thrombocythemic onset successfully treated with antegrade intraarterial thrombolytic therapy followed by anticoagulant therapies. ( info) |
| paraneoplastic syndromes including leukocytosis, thrombocytosis and hypercalcemia are occasionally seen in patients suffering from progressive malignant disorders. Recent studies have revealed the production of several humoral factors by tumor cells and normal splenic cells of tumor-bearing patients to be the major cause of these reactions. granulocyte-macrophage colony-stimulating factor (GM-CSF), granulocyte-colony stimulating factor (G-CSF), parathyroid hormone-related peptide, interleukin (IL)-1, IL-6, and tumor necrosis factor (TNF) have been implicated. We describe a 58-year-old Japanese man with squamous cell carcinoma (SCC) on the left sole, which developed in a deep linear scar after a train crash. He developed pulmonary and lymph node metastases, then leukocytosis (57,110/mm3 with 95% neutrophilia), thrombocytosis (86.3 x 10(4)/mm3), and hypercalcemia (7.0 mEq/1), and finally cachexia, followed by death. serum G-CSF, IL-1 alpha, IL-1 beta, and TNF-beta were determined; revealing G-CSF and IL-1 beta levels were above the upper limits of their normal ranges at 39.2 pg/ml and 4.63 pg/ml, respectively. It is probable that these humoral factors were partially responsible for the paraneoplastic syndromes induced by the cutaneous SCC with metastasis in the present case. ( info) |
4/303. Clinical implications of elevated PAI-1 revisited: multiple arterial thrombosis in a patient with essential thrombocythemia and elevated plasminogen activator inhibitor-1 (PAI-1) levels: a case report and review of the literature. Plasminogen activator inhibitor (PAI-1), a member of the serine protein family, is the most active in vivo inhibitor of fibrinolysis induced by plasminogen, tissue plasminogen activator (tPA), and urokinase type plasminogen activator (uPA). While the association between elevated PAI-1 and thrombogenesis has been well studied for several disease processes, including coronary disease, postoperative deep vein thrombosis (DVT), myocardial infarction, malignancy, and diabetes, few studies have concentrated on the correlation between elevated PAI-1 levels and thrombogenesis in patients with myeloproliferative disorders. Essential thrombocythemia (ET), a chronic myeloproliferative disorder, characterized by the overproduction of poorly functioning platelets, is associated with both thrombotic and hemorrhagic life-threatening complications. Although the events resulting in thrombogenesis in such patients may be multifactorial in nature, an association between elevated PAI-1 levels and thrombus formation has been proposed. Herein we present a patient diagnosed with ET complicated by multiple episodes of arterial thrombosis. Elevations in PAI-1 levels were documented repeatedly. The role of elevated PAI-1 when associated with other disease processes is also discussed. ( info) |
5/303. hydroxyurea-induced leg ulcers: is macroerythrocytosis a pathogenic factor? hydroxyurea is a common cancer chemotherapy agent that inhibits ribonucleotide reductase, an enzyme essential to dna synthesis. It is considered the drug of choice in the treatment of chronic myelogenous leukemia and essential thrombocythemia. The occurrence of leg ulcers have been described in 8.5% of patients receiving continuous treatment with hydroxyurea, but the cause of this complication is unknown. We report two additional patients and suggest that macroerythrocytosis, which occurs in almost all the patients taking hydroxyurea, may be a pathogenic factor. Macroerythrocytosis can be considered as an 'acquired' blood dyscrasia, and similar leg ulcers have long been known to occur with certain hereditary blood dyscrasias, such as sickle cell anemia, thalasemia, and spherocytosis. ( info) |
6/303. thrombocytosis with sideroblastic erythropoiesis: a mixed myeloproliferative myelodysplastic syndrome. Some patients with haematological neoplasms have features which overlap between a myelodysplastic syndrome and a myeloproliferative disorder. Two such patients are reported, both having sideroblastic erythropoiesis and thrombocytosis and one sequentially developing features of atypical chronic myeloid leukaemia, idiopathic myelofibrosis and acute megakaryoblastic leukaemia. The prevalence of thrombocytosis among cases of refractory anaemia with ring sideroblasts may be as high as 15-20% and has implications for choice of therapy. ( info) |
7/303. Lessons to be learned: a case study approach: pseudohyperkalaemia due to thrombocytosis in a case of tubo-ovarian abscess. The case described here is that of a 48-year-old lady who presented with abdominal pain and fever; at a later stage she was found to have hyperkalaemia of uncertain origin. blood examination revealed there to be marked elevation of the platelets (thrombocytosis) on some occasions. It was then realised that there was correlation between the platelet levels and serum potassium values. During the clotting process the release of potassium from the increased number of platelets caused the serum potassium to be elevated on account of an in vitro effect. The important point is that the raised serum potassium levels were not due to an in vivo phenomenon and, therefore, the patient did not need treatment for this; however, the presence of thrombocytosis was itself a clue to the diagnosis--which was eventually recognised as being due to an infection. At operation a tubo-ovarian abscess was discovered to be the cause of the problem. ( info) |
8/303. Translocation (X;5)(q13;q33) in essential thrombocythemia. We report a novel chromosomal translocation (X;5)(q13;q33) in a woman with no history of prior chemotherapy or radiotherapy, found to have essential thrombocythemia. Aberrations in chromosome 5, mostly deletions of 5q, have been described in essential thrombocythemia; however, a t(X;5) translocation has not been reported. ( info) |
9/303. Acute myocardial infarction following toxic epidermal necrolysis? We describe a 29-year-old woman with rheumatoid arthritis who suffered an acute myocardial infarction 70 days after an initial presentation with toxic epidermal necrolysis (TEN). The trigger for the TEN was probably an over-the-counter anti-influenza treatment containing tipepidine hibenzate. Although the patient had familial hypercholesterolemia, we believe that thrombocytosis, induced by the inflammatory response and metabolic stress resulting from the TEN, may also have played a significant role in the pathogenesis of the myocardial infarction. Although TEN manifests itself principally as a skin disease, the potential for systemic morbidity, including cardiovascular abnormalities, should also be remembered. ( info) |
10/303. Longitudinal melanonychia associated with hydroxyurea therapy in a patient with essential thrombocytosis. Occurrence of skin lesions during long-term therapy with hydroxyurea are well described, but there have been only a few case reports of longitudinal melanonychia associated with hydroxyurea treatment. We report herein longitudinal melanonychia associated with hydroxyurea therapy in a subject with essential thrombocytosis. The prolonged time lapse (1 year) between initiation of hydroxyurea therapy and the onset of the longitudinal melanonychia should be noted. ( info) |